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Case Study: July 2007
History
A 55 year old presents with brownish deposits in both corneas. A biopsy of corneal epithelium and superficial stroma are submitted to the ophthalmic pathology laboratory. A diagnosis of atypical melanosis is queried clinically.
Figure 1 shows the H and E
Figure 2 shows a Masson’s trichrome stain.
Figure 3 is higher power of figure 2.
Questions
1. What do Figures 1 and 2 show ?
2. What is the likeliest diagnosis?
3. Which further stains could be performed in this case?
4. Which other tests may you wish to know the results of?
1. Figure 1 shows eosinophilic rhomboid-shaped crystals in the cytoplasm of the corneal epithelial cells. Figure 2 shows that the crystals stain crimson with a Masson’s Trichrome. No crystals were present in the stroma.
2. Given the shape of the crystals and their reactivity with Masson’s Trichrome, the diagnosis is of paraproteinaemia or monoclonal gammopathy corneal crystalline keratopathy.
3. Immunohistochemistry for immunoglobulins and light chains.
4. Haematological investigations (film, marrow) and X-ray investigations.
In this case, the patient had established myeloma (I hid that bit of the history !!).
Copper can also deposit in the cornea in the setting of a monoclonal gammopathy.
References
Garg S, Jampol LM, Lewis RA, Penner JA.
Corneal copper deposition secondary to a variant of multiple myeloma: 30-year catamnesis.
Arch Ophthalmol. 2006 Jan;124(1):130-2.
Hawkins AS, Stein RM, Gaines BI, Deutsch TA.
Ocular deposition of copper associated with multiple myeloma.
Am J Ophthalmol. 2001 Feb;131(2):257-9.
Garibaldi DC, Gottsch J, de la Cruz Z, Haas M, Green WR.
Immunotactoid keratopathy: a clinicopathologic case report and a review of reports of corneal involvement in systemic paraproteinemias.
Surv Ophthalmol. 2005 Jan-Feb;50(1):61-80. Review
Buerk BM, Tu EConfocal microscopy in multiple myeloma crystalline keratopathy. Cornea. 2002 Aug;21(6):619-20.
Kato T, Nakayasu K, Omata Y, Watanabe Y, Kanai A.
Corneal deposits as an alerting sign of monoclonal gammopathy: a case report.
Cornea. 1999 Nov;18(6):734-8.
Hutchinson K, Dal Pra M, Apel A.Immunoglobulin G crystalline keratopathy associated with monoclonal gammopathy.
Perry HD, Donnenfeld ED, Font RL.
Intraepithelial corneal immunoglobulin crystals in IgG-kappa multiple myeloma. Cornea. 1993 Sep;12(5):448-50.
Steuhl KP, Knorr M, Rohrbach JM, Lisch W, Kaiserling E, Thiel HJ.
Paraproteinemic corneal deposits in plasma cell myeloma.
Am J Ophthalmol. 1991 Mar 15;111(3):312-8.
Beebe WE, Webster RG Jr, Spencer WB.
Atypical corneal manifestations of multiple myeloma. A clinical, histopathologic, and immunohistochemical report. Cornea. 1989 Dec;8(4):274-80.
Ormerod LD, Collin HB, Dohlman CH, Craft JL, Desforges JF, Albert DM.
Paraproteinemic crystalline keratopathy. Ophthalmology. 1988 Feb;95(2):202-12. Review.
